Marfan syndrome, an insidious disease with an unexpected course

Introduction: The study presents a 45-year-old male with Marfan syndrome admitted to the cardiac surgery department with a suspected acute dissecting aneurysm of the ascending aorta involving distal thoracic and abdominal aorta. Case report: Patient was qualified for the surgical intervention and the Bentall procedure with valve On-X and ascending aorta replacement with vascular prosthesis Gelweave was performed. Patient was readmitted 8 months later for reoperation due to the progress of the aortic arch aneurysm. During the redo surgery mediastinal pseudoaneurysm originating from the anastomosis between vascular prosthesis and aortic arch was identified. Aortic arch was replaced with vascular prosthesis Thoraflex. After 3 months the patient presented chronic cardiac tamponade decompressed with substernal surgical access. 1 month later patient was readmitted with pseudoaneurysm of the ascending aorta. Supracoronary section of the ascending aorta was replaced with a new Gelweave vascular prosthesis and the bleeding that originated from the left coronary artery ostium was treated accordingly. Marfan syndrome require multidisciplinary care and often multistage risk surgical treatments, including postoperative bleeding or pseudoaneurysms. There are no clear data on the choice of valve prosthesis. Each time, the surgeon must center the risk of hemorrhagic complications against the necessity for reoperation due to degeneration of the biological prosthesis.